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MRS features during encephalopathic crisis period in 11 years old case with GA-1

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Date
2015-05-01
Author
AKSU, Banu
Alkan, ALPAY
Iscan, AKIN
Kurtcan, SERPİL
GULER, Serhat
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Type
Article
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Abstract
Glutaric aciduria type-1 (GA-1) is a disorder of amino acid metabolism. The usual clinical-onset is an acute encephalopathic crisis in early childhood. There are only a few cases diagnosed in older age groups. MRI features of the disease are well defined. However, there are limited number of studies concerning advanced neuroimaging findings. We present DWI and MRS findings of an 11 year-old GA-1 patient admitted with an encephalopathic crisis. Diffusion restrictions in bilateral basal ganglia, corpus callosum and periventricular deep white matter were observed. In left occipital periventricular white matter and left basal ganglia, mild increased Cho/Cr and MI/Cr ratios and decreased NAA/Cr ratio were detected. Also inverted double lactate peak (TB: 135 ms) was present at 1.33 ppm in the left basal ganglia. In addition to these findings, a peak at 1.56 ppm above the baseline was seen on both short and long echo-time MRS in left occipital lobe deep white matter which may show accumulation of degradation products of amino acids in the GCDH enzyme deficiency. (Cd) 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Subject
Kurtcan S., AKSU B., Alkan A., GULER S., Iscan A., -MRS features during encephalopathic crisis period in 11 years old case with GA-1-, BRAIN & DEVELOPMENT, cilt.37, ss.546-551, 2015
URI
https://hdl.handle.net/20.500.12645/8976
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BEZMIALEM VAKIF UNIVERSITY

About us |Policies | Library | Contact us | Send Feedback | Sitemap | Admin

Bezmialem Vakıf Üniversitesi, Adnan Menderes Bulvarı Vatan Caddesi 34093 Fatih, İstanbul / TURKEY
Copyright © Bezmialem Vakıf Üniversitesi

Creative Commons Lisansı
Bezmialem Institutional Repository, Creative Commons Alıntı-GayriTicari-Türetilemez 4.0 Uluslararası Lisansı ile lisanslanmıştır.

OpenAccess@BVU

Support by  UNIREPOS